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Blood, 15 October 2008, Vol. 112, No. 8, pp. 3082-3087. Prepublished online as a Blood First Edition Paper on July 22, 2008; DOI 10.1182/blood-2008-05-154609.
CLINICAL TRIALS AND OBSERVATIONS Long-term outcomes of patients with advanced-stage cutaneous T-cell lymphoma and large cell transformation1 Division of Haematology and Medical Oncology, Peter MacCallum Cancer Centre, Melbourne; 2 Monash University, Clayton; 3 University of Melbourne, Parkville; 4 Department of Dermatology, St Vincent's Hospital Department of Medicine, Melbourne; and 5 Department of Pathology and 6 Division of Radiation Oncology, Peter MacCallum Cancer Centre, Melbourne, Australia Although mycosis fungoides (MF) is typically an indolent disease, patients with advanced-stage disease (stages IIB-IVB), including Sézary syndrome (SS), often have a poor outcome. A 31-year, retrospective analysis of our cutaneous lymphoma database, of 297 patients with MF and SS, was undertaken to study long-term outcomes and identify clinical predictors of outcome in patients with advanced-stage disease (ASD, n = 92) and large cell transformation (LCT, n = 22). Two-thirds of patients with ASD presented with de novo ASD. The median overall survival (OS) for ASD was 5 years with a 10-year predicted OS of 32%. Age at initial diagnosis (P = .01), tumor stage (P = .01), and clinical stage (P = .001) were found to be significant predictors of outcome. Patients who presented with de novo ASD demonstrated better outcomes that were not statistically significant than those with a prior diagnosis of early-stage MF (P = .25). Transformation developed in 22 of the 297 MF/SS patients (7.4%), with a transformation rate of only 1.4% in patients with early-stage disease, compared with stage IIB (27%) and stage IV (56%-67%) disease. The median OS from diagnosis of LCT was 2 years. We confirm that the incidence of LCT is strongly dependent on tumor stage at diagnosis, and we demonstrate a much lower overall risk of LCT than previously reported.
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